Morvan’s syndrome—is a pathogen behind the curtain?

Singh, R.N.; Das, Pritam; Kaur, Upinder; Misra, Anamika; Choudhury, Ashis Kumar; Manna, Sukdev; Gaude, Rohit; Gautam, Deepak; Gambhir, Indrajeet Singh; Chakrabarti, Sankha Shubhra

doi:10.1007/s10072-018-3515-y

Cited by 13 publications

(10 citation statements)

References 18 publications

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“…However, Irani et al [ 7 ] reported that 3 of 29 patients with Morvan syndrome did not have Caspr2 or LGI1 positivity and suggested that some patients may have antibodies against other VGKC-complex (or uncomplexed) antigens. There are no clear triggers for Morvan syndrome [ 8 ], although a series of 4 patients described in India in 2017 suggested a possible viral trigger [ 9 ], as suspected in our patient.…”

Section: Discussionmentioning

confidence: 67%

A Patient with Double-Negative VGKC, Peripheral Nerve Hyperexcitability, and Central Nervous System Symptoms: A Postinfectious Autoimmune Disease

Eikeland¹

2020

Case Reports in Neurological Medicine

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Research in the last few years has indicated that most voltage-gated potassium channel- (VGKC-) complex antibodies without leucine-rich glioma-inactivated protein 1 or contactin-associated protein-like 2 antibody specificity lack pathogenic potential and are not clear markers for autoimmune inflammation. Here we report on a patient with double-negative VGKC who developed severe peripheral nerve hyperexcitability, central nervous system symptoms with agitation and insomnia, dysautonomia, and systemic symptoms with weight loss, itch, and skin lesions. The disease started acutely one month after an episode of enteroviral pericarditis and responded well to immunotherapy. The patient is presumed to have developed a postinfectious immunotherapy-responsive autoimmune disease. In the setting of anti-VGKC positivity, it seems likely that anti-VGKC contributed to the pathogenesis of the patient’s symptoms of nerve hyperexcitability and that the disease was caused by an acquired autoimmune effect on the neuronal kinetics of VGKC. It is still unknown whether or not there are unidentified extracellular molecular targets within the VGKC-complex, i.e., a novel surface antigen and a pathogenic antibody that can cause affected individuals to develop a peripheral nerve hyperexcitability syndrome. This case highlights the fact that less well-characterized autoimmune central and peripheral nervous system syndromes may have infectious triggers.

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Section: Discussionmentioning

confidence: 67%

A Patient with Double-Negative VGKC, Peripheral Nerve Hyperexcitability, and Central Nervous System Symptoms: A Postinfectious Autoimmune Disease

Eikeland¹

2020

Case Reports in Neurological Medicine

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“…to several arthropod-borne viral fever syndromes. The diagnostic facilities are nonexistent in the periphery, and the majority of these infections may be subclinical or may present with atypical manifestations [22]. An idea can be obtained from the data of the Integrated Disease Surveillance Programme (IDSP) of the Ministry of Health and Family Welfare (MoHFW) of the government of India.…”

Section: Covid-19 Mortality-why the Indian Cloud Seems To Have A Silvmentioning

confidence: 99%

COVID-19 in India: Are Biological and Environmental Factors Helping to Stem the Incidence and Severity?

Chakrabarti¹,

Kaur²,

Banerjee³

et al. 2020

Aging and disease

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The ongoing Corona virus (COVID-19) pandemic has witnessed global political responses of unimaginable proportions. Many nations have implemented lockdowns that involve mandating citizens not to leave their residences for non-essential work. The Indian government has taken appropriate and commendable steps to curtail the community spread of COVID-19. While this may be extremely beneficial, this perspective discusses the other reasons why COVID-19 may have a lesser impact on India. We analyze the current pattern of SARS-CoV-2 transmission, testing, and mortality in India with an emphasis on the importance of mortality as a marker of the clinical relevance of COVID-19 disease. We also analyze the environmental and biological factors which may lessen the impact of COVID-19 in India. The importance of cross-immunity, innate immune responses, ACE polymorphism, and viral genetic mutations are discussed.

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“…Early on, the clinical course of the patient resembled that of anti-NMDA receptor encephalitis. Although, both anti-NMDA receptor encephalitis and Morvan’s syndrome can have a variable prognosis,5 many patients, such as ours can recover surprisingly well despite severe symptoms and a prolonged disease course.…”

Section: Discussionmentioning

confidence: 82%

“…Despite the lack of randomised controlled trials, we believe this case illustrates the importance of aggressive treatment. Rituximab has been used in the past for treatment of postviral Epstein-Barr virus autoimmune haemolytical anaemia and a recent paper by Singh et al suggested a probable viral prodrome for illnesses like Morvan’s syndrome 5…”

Section: Discussionmentioning

confidence: 99%

Morvan’s syndrome treated successfully with rituximab and lacosamide

Sveinsson¹,

Nimer

Piehl

2019

BMJ Case Rep

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We describe a woman with both central and peripheral nervous system symptoms consistent with Morvan’s syndrome who was successfully treated with immunosuppression including rituximab and the new antiepileptic drug lacosamide against peripheral nerve hyperexcitability. Despite being over 8 months in hospital and 4 months in an intensive care unit she recovered fully. It is also the first case where cerebrospinal fluid neurofilament-light (NfL) levels were followed during the disease course. The clinical course resembled that of anti-NMDA receptor encephalitis, where patients often recover surprisingly well despite severe symptoms and an extensive time in intensive care. A possible explanation is the comparatively low levels of NfL, indicating disease processes that are not characterised by extensive neuroaxonal degeneration.

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Morvan’s syndrome—is a pathogen behind the curtain?

Cited by 13 publications

References 18 publications

A Patient with Double-Negative VGKC, Peripheral Nerve Hyperexcitability, and Central Nervous System Symptoms: A Postinfectious Autoimmune Disease

A Patient with Double-Negative VGKC, Peripheral Nerve Hyperexcitability, and Central Nervous System Symptoms: A Postinfectious Autoimmune Disease

COVID-19 in India: Are Biological and Environmental Factors Helping to Stem the Incidence and Severity?

Morvan’s syndrome treated successfully with rituximab and lacosamide

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